Is the SHRPS strain a suitable model of spontaneous CADASIL?

Silvana Penco, Paolo Gelosa, Silvana Pileggi, Mauro Abbate, Alessandro Marocchi, Uliano Guerrini, Alice Pignieri, Elena Tremoli, Luigi Sironi

Research output: Contribution to journalArticle

Abstract

A number of features of the pathology occurring in spontaneously hypertensive stroke prone rats (SHRSPs), such as MRI brain signal abnormalities, the presence of high protein content in cerebrospinal fluid and vessel wall thickening, seem to indicate that this strain is a suitable model for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). To explore this hypothesis, we sought the human diagnostic hallmarks of the disease [the accumulation of granular osmiophilic material (GOM) deposits in vessel walls and NOTCH3 gene mutations] in SHRSPs. Male SHRSPs fed a permissive diet were sacrificed 3 days after the first MRI visualisation of brain abnormalities. Whole blood and kidney samples were respectively collected for molecular and electron microscopy evaluations. Automated sequence analysis of exons and intron-exon boundaries did not reveal any genetic variation in the NOTCH3 gene, and electron microscopy excluded the presence of GOM. The findings of this study exclude SHRSPs as a possible model for CADASIL.

Original languageEnglish
Pages (from-to)427-430
Number of pages4
JournalJournal of Molecular Neuroscience
Volume46
Issue number2
DOIs
Publication statusPublished - Feb 2012

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CADASIL
Stroke
Exons
Electron Microscopy
Brain
Introns
Genes
Sequence Analysis
Cerebrospinal Fluid
Pathology
Diet
Kidney
Mutation
Proteins

ASJC Scopus subject areas

  • Medicine(all)

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Is the SHRPS strain a suitable model of spontaneous CADASIL? / Penco, Silvana; Gelosa, Paolo; Pileggi, Silvana; Abbate, Mauro; Marocchi, Alessandro; Guerrini, Uliano; Pignieri, Alice; Tremoli, Elena; Sironi, Luigi.

In: Journal of Molecular Neuroscience, Vol. 46, No. 2, 02.2012, p. 427-430.

Research output: Contribution to journalArticle

Penco, Silvana ; Gelosa, Paolo ; Pileggi, Silvana ; Abbate, Mauro ; Marocchi, Alessandro ; Guerrini, Uliano ; Pignieri, Alice ; Tremoli, Elena ; Sironi, Luigi. / Is the SHRPS strain a suitable model of spontaneous CADASIL?. In: Journal of Molecular Neuroscience. 2012 ; Vol. 46, No. 2. pp. 427-430.
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